Thoughts about Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease

If it were to be known that spongiform encephalopathy, is epidemic in the United States, the economic meltdown and consequences would be immense. Which would be detrimental to our dying economy, and to the powers that be. As well as substantially dislocating major segments of the rendering and livestock industries):

It would appear that spongiform encephalopathy is now rampant in our wildlife populations. In 1997 Doctors in Kentucky issued a warning that people should not eat squirrel brains, a regional delicacy, because squirrels may carry a variant of mad cow disease that can be transmitted to humans and is fatal.

Moose, elk, deer, mink, rabbits, raccoons, skunks, and other wild animals are known to develop variants of mad cow disease that collectively are called transmissible spongiform encephalopathies.

In the last years, there have been many human cases of a form of transmissible spongiform encephalopathy, called Creutzfeldt-Jakob disease. No matter what you may call it, they are all killer prions.

Prions are small, proteinaceous infectious particles that contain no detectable nucleic acid of any form, but are transmissible among certain animals, where they cause fatal brain diseases. These particles are rod-shaped, about 165 nanometers long and about 11 nanometers in diameter, and they consist largely of a protein called PrPSc, having molecular weight 33,000-35,000. They are able to resist inactivation by boiling, acid (pH 3-7), ultraviolet radiation (254 nm), formaldehyde, and nucleases! They can be inactivated by boiling in detergents, alkali (pH > 10), autoclaving at 132 degrees centigrade for over 2 hours, and denaturing organic solvents such as phenol.

Stanley Prusiner won the Nobel prize for medicine in 1997 for his work on prions. His theory is that prions are a modified form of a protein naturally occuring in the brain (PrP), and that this modified form can arise from a cell mutation, but then spread by means of a kind of autocatalyzed chain reaction. This theory was initially very controversial, because all other self-reproducing biological entities appear to contain RNA or DNA. There are still many doubters. In the earlier literature prions are sometimes called "slow viruses", because of their slow effect. However, no virus has ever been associated with prion diseases.


While the victims could have contracted the disease from eating beef and not wild game, authorities have good reason to be reluctant to confirm a case of mad cow disease here in the United States. Since it is said that almost all of the known victims with the disease had contact with meat from wild game.


Attack of the killer prions

By Mary Rodgers

How’s this for a B-movie plot? Prions, killer proteins that can’t be destroyed, either by blistering heat or by any known disinfectant, turn good proteins bad. The bad proteins enter the food chain when infected animals are processed into feed that is foisted on captive herbivores. Now picture mad scientists devising more ways to turn cows into carnivores and ask yourself, “Why on earth?” Cut to greedy factory-farm execs looking to turn waste products into profits, and there’s your answer.


Who can you trust?





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